PRCA facts

Question 1

Transient aplastic crisis

Answer 1

Parvo B19
Predisposing factors of immunodeficiency (HIV/SCID) or hereditary hemolytic anemia (Spherocytosis…)
In immunodeficiency may need Tx with IVIG
In congenital hemolysis syndromes- no need for Tx

Question 2

Diamond- Blackfan anemia

Answer 2

Hereditary PRCA

Question 3

Good syndrome

Answer 3

Hypoglobulinemia and thymoma
May present with PRCA, LGL

Question 4

Diseases associated with PRCA

Answer 4

T-LGL
Lymophoma (CLL)
Thymoma
Good syndrome
Post ABO incompatible all transplant

Question 5

BM diagnosis of PRCA

Answer 5

Erythroid hypoplasia(<1-5%)
With giant erythroblast
Vacualted

Question 6

DD of PRCA

Answer 6

Copper deficiency
MDS
AA

Question 7

w/u of PRCA

Answer 7

Anemiogram including EPO and Coombs
Erytorocyte adenosine deaminase and genetic testing for Diamond-Blackfan anemia
FACS, PCR TCR and STAT3 mutation for LGL
ANA/RF
PEP/IEP/FLC
Parvo, HBV, HCV, HIV, EBV, CMV
BMB
CT scan of chest for thymoma

Question 8

Testing for Parvo B19

Answer 8

PCR
IgM may aid with Dx
Positive IgG excludes Dx except in the context of HIV/SCID

Question 9

1st line Tx of PRCA

Answer 9

Steroids+-
CsA

Question 10

Time to response in PRCA

Answer 10

Usually 2-3 months after initiation of Tx

Question 11

Duration of Tx in PRCA

Answer 11

1-2 months after normalization of Hb
(total of 3-6 months)

Question 12

CsA effectiveness in PRCA

Answer 12

70-80% ORR

Question 13

Maintenance Tx in PRCA

Answer 13

Low dose CsA
Combination with steroids improves relapse free survival

Question 14

1st line Tx of PRCA when CsA contraindications

Answer 14

Cyclophosphamide or MTX +- Steroids

Question 15

CY effectiveness in PRCA

Answer 15

40-60%
Median time of 12 weeks
Tx not exceeding 6 months
Mainly effective in LGL associated PRCA

Question 16

MTX in T-LGL associated PRCA

Answer 16

Less effective than CY
Causes anemia
Mainly effective in T-LGL with neutropenia

Question 17

Thymoma frequency in PRCA

Answer 17

30%

Question 18

PRCA frequency in thymoma

Answer 18

5-7%

Question 19

2nd line Tx for PRCA

Answer 19

Alemtuzumab
Anti-CD52
low dose of 10mg for 10 days or 10 mg 1/w for 4/8 weeks
PCP HSV prophylaxis
IVIG before Tx if hypogamma

Question 20

2nd line Tx for PRCA- other options

Answer 20

Sirolimus
ORR 80% in 2nd line
ORR 90% in 1st line (eg. CKD pts)

Question 21

Tx of PRCA in RA pts

Answer 21

Tofacitinib

Question 22

Tx of PRCA in CLL pts

Answer 22

Rituximab

Question 23

Tx of PRCA in MGUS pts

Answer 23

Bortezomib
Rituximab (if IgM)

Question 24

PRCA developing in low risk MDS on EPO

Answer 24

May be due to Ab to EPO
Related ro SC administration of some products